During 2012, projects started in the UK looking at Canine Epileptoid Cramping Syndrome (CECS). CECS is the terminology used, as it has come to be accepted but it must be stressed once more this is simply a descriptive name that broadly fits a range of presenting signs. Last year's report mentioned the interest being shown by veterinary neurologists based in the UK and this report updates readers about progress.
Two separate investigations were started during the year. One, recommended by the Border Terrier Club and myself, is based at the Royal Veterinary College in London under the direction of Dr Holger Volk. This group are looking for descriptions of the symptoms associated with cases of CECS along with any factors thought to trigger such episodes. Their ambition is to identify a common pattern of symptoms that could be useful in the diagnosis of CECS.
The second group are being more ambitious in seeking not only to characterise the symptoms but also to move forward to attempt to identify the inheritance and the genes responsible. This study is being led by Dr Laurent Garosi with an ultimate ambition of progressing towards a suitable gene test. His work is based upon a theory that CECS may have some commonality with neurological conditions seen in other breeds. Dr Garosi and I had the opportunity to discuss this study and co-operation with the study is now recommended. Both groups have finished collecting their data with the first asking for information from Border Terrier owners and the second collecting data and samples from the owner's veterinary surgeon. For both projects the number of cases coming forward is relatively small and we await the outcome of each. However Dr Garosi has provided an interim report to a meeting of veterinary neurologists in Ghent this year. Perhaps the abstract from his report will be of interest. I have added a few words to explain some of the technical terms.
A disorder referred to as canine epileptoid cramping syndrome (CECS) has been observed in Border Terriers (BT). Episodes consist of an inability to stand or walk, involuntary flexion or extension of one or multiple limbs while maintaining consciousness during the episode. An owner-directed survey was conducted to characterize the phenotype (presentation of the symptoms -spd) and identify possible precipitating factors. BT with at least a 12-month history of episodes of abnormal involuntary hyperkinetic movement (unusual, increased movement - spd) or muscle tone, without autonomic signs (unconscious activity) or loss of consciousness were included on the basis of video recording review of the episodes (15/29) or the owner's detailed description of the episodes (14/29). Twenty-nine BT met these inclusion criteria. Age of onset ranged from 10 weeks to 7 years of age (mean and median of 3 years). Most affected dogs had their first episode before 3 years of age and there was no sex predisposition. The majority of episodes lasted from 2 to 30 minutes (25/29) [ranging from 30 seconds to 2 ½ hours]. Episodes occurred as often as several per day (16/29) or infrequently with several months or years between episodes. Overall frequency was very variable between dogs and for a given dog, with the majority of owners (15/29) reporting clusters over a few days interspaced by weeks or months of normality. The majority of owners (18/29) felt that they could predict an episode, with dogs becoming quieter (11/29), seeking to be near owners (6/29), vomiting bile or eating grass (4/29) shortly before onset. None of the owners felt they could stop the episode. The most frequent observations during the episodes were difficulty walking (27/29), mild tremor (21/29) and dystonia (22/29) (involuntary muscle spasms - spd). Episodes affected all four limbs (25/29), head and neck (21/29); with back and abdomen (16/29) and tail (11/29) less frequently involved. Borborygmi (grumbling noises from the bowel - spd) were reported in 11/29 dogs. Although the majority of episodes appeared to be totally random (16/29) owners identified waking up from sleep (13/29), excitement (10/29), stress (9/29), hot/cold temperatures (7/29), as precipitating factors. 14/29 dogs were reported to have episodes of vomiting and diarrhoea with half of these being linked to the occurrence of episodes of CECS (7/14). Most owners (26/29) had changed their dog's diet after a presumptive diagnosis of CECS was made, with over half (14/26) reporting an improvement in frequency of the episode following this. Therapeutic trials with phenobarbitone (5/29), potassium bromide (2/29), diazepam (3/29), and buscopan (3/29) failed in all dogs tested. This survey may provide the basis for candidate gene investigation and for further study of the role of diet and gastro-intestinal involvement for this condition.
One characteristic that appears to be common in similar conditions reported across other breeds is how the patient retains consciousness. This is thought to generally help differentiate these conditions from epilepsy. Several breeds are mentioned in the literature with Cavaliers, Border Terriers, Cairns, Scotties and Norwich Terriers being just examples. However this list is not exhaustive. In each, symptoms include a degree of involuntary muscle spasm, loss of ability to control the limbs but no loss of awareness. Such episodes may be frightening to watch but are not considered life-threatening for the affected dog. As examples, episodic falling syndrome in Cavaliers is triggered by stress or excitement. Scottie cramp seems to lock up the muscles of the hind and forelimbs causing dogs to walk with a stilted gait or they fall over. In each of these conditions it is suspected there is a deficiency in the chemical secretions that control and modulate muscle contractions. Research has identified the marker genes for episodic falling and we can hope for a similar breakthrough for CECS.
Success will rely upon accurate identification of cases through diagnosis and this is why two groups have taken an interest in the border terrier. A good diagnosis is necessary if there is to be hope of identifying a mutation that will lead to a genetic test. If we are fortunate and such a test is possible we will need to work together as a breed to reduce and then eliminate the mutation. However we are some distance away from that possibility.
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